Familial Waldenstrom’s macroglobulinemia and relation to immune defects, autoimmune diseases, and haematological malignancies – A population-based study from northern Sweden

Abstract

The incidence of WM in Norrbotten and Västerbotten counties was higher than expected. We found a strong correlation between autoimmune/inflammatory diseases, other haematological malignancies, and familial WM and a high frequency of serum immunoglobulin abnormalities in the relatives of the WM patients, findings that strengthen the hypothesis that the aetiology of WM depends on both immune-related and genetic factors.

Prognostic factors and primary treatment for Waldenström macroglobulinemia – a Swedish Lymphoma Registry study

“…Waldenström macroglobulinaemia is a rare disease with a worldwide incidence of 3–4 million persons per year and this rate varies between geographical areas: US = 3·8 (Wang et al , ), South East England = 5·5 (Phekoo et al , ) and Japan = 0·43 (Iwanaga et al , ). We observed a three times higher incidence of WM in Sweden (11·5 per million persons per year) and an even higher incidence in northern Sweden (Brandefors et al , ). Contributing factors to this could be that Sweden has a mandatory cancer registry and a high proportion of WM patients with asymptomatic disease at diagnosis are followed with a “watch and wait” policy (73·4%), while the corresponding figure in the literature is approximately one‐quarter of the patients (Pophali et al , ).…”

“…The incidence of WM has geographical, ethnic and gender differences and there are associations with immune conditions and family history of haematological malignancy for the development of WM (Ekström Smedby et al , ; Kristinsson et al , , ). We have observed aggregations of families with WM and a co‐occurrence with autoimmune diseases in the northern Sweden (Brandefors et al , ), a region with a small but stable and, in some areas, isolated population. This suggests that both genetic and environmental factors can influence the incidence of disease.…”

“…Waldenstr€ om macroglobulinaemia (WM)/lymphoplasmacytic lymphoma (LPL) is a rare disease, with a worldwide incidence of 3-4 per million persons per year (Wang et al, 2012;Brandefors et al, 2016). The diagnosis of WM requires infiltration of LPL cells in the bone marrow and any concentration of IgM monoclonal immunoglobulin (MI) (Owen et al, 2003;Swerdlow et al, 2008).…”

Incidence and inheritance of hyperphosphorylated paratarg-7 in patients with Waldenstrom’s macroglobulinaemia in Sweden

“…WM is associated with immune conditions such as autoimmune diseases and specific infections, but this association is not found in MM [7,8]. Using the Swedish Lymphoma Registry, we found a high incidence of WM in northern Sweden with aggregations of families with WM and a co-occurrence with autoimmune diseases and other haematological malignancies in 75% and 42% of the families, respectively [9].…”

“…In Sweden, the incidence of WM/LPL is high; 11.5, measured as million persons per year, with an even higher incidence in the northern counties; Norrbotten County = 17.6; and Vasterbotten County = 14.3 million persons per year [17] and with an aggregation of families in these counties. This high incidence and family aggregation in one region may relate to heredity, but environmental and other unknown factors may also contribute [9].…”